Cushing’s syndrome complicated by tumors secreting 2 hormones
Woman with Cushing's due to cortisol also had high aldosterone levels
Cushing’s syndrome in a 45-year-old woman was complicated by primary aldosteronism, the result of tumors in the adrenal glands secreting both cortisol and aldosterone, according to a case reported in China.
Aldosteronism occurs when the adrenal glands produce high levels of aldosterone, a hormone that regulates blood pressure and balances sodium and potassium levels.
In this case, the patient had a tumor secreting both these hormones in the right adrenal gland, and a cortisol-producing tumor and an aldosterone-producing nodule in the left adrenal gland.
“This case suggests that it is necessary to assess the presence of [primary aldosteronism], even in [Cushing’s syndrome] with apparent symptoms. As patients with [both conditions] may have more complicated adrenal lesions, more data are required for diagnosis,” the researchers wrote.
Cushing’s syndrome complicated by primary aldosteronism not ‘well known’
The report, “Bilateral co-secretory lesions presenting with coexisting Cushing syndrome and primary aldosteronism: a case report,” was published in the journal BMC Endocrine Disorders.
Cushing’s syndrome includes a group of conditions characterized by excessive levels of cortisol. When the disease is caused by a tumor in the brain’s pituitary gland that increases adrenocorticotropic hormone (ACTH) levels, stimulating cortisol production by the adrenal glands, it is known as Cushing’s disease.
Tumors in the adrenal glands that sit atop the kidneys, however, also can lead to excessive cortisol production.
Although reports are rising of primary aldosteronism in people with asymptomatic Cushing’s syndrome, “[primary aldosteronism] screening may be ignored in the diagnosis and treatment of adrenal Cushing’s syndrome,” the researchers wrote.
The potential for primary aldosteronism to be complicated by aldosterone- and cortisol-producing tumors is known, but “cases of apparent Cushing’s syndrome complicated with [primary aldosteronism] are less well known,” they added.
Researchers at West China Hospital of Sichuan University described the case of a woman who developed Cushing’s syndrome and primary aldosteronism due to tumors in the adrenal glands.
The woman had high blood pressure, or hypertension, with weight gain over one year and facial swelling for six months. When admitted to the hospital’s adrenal disease center, she was overweight, had a moon-shaped face, thin skin, and fat accumulation on the back of the neck.
Laboratory tests showed that she had lost the daily rhythms of cortisol production, normally characterized by a peak in the morning and low levels at night. A dexamethasone test — which triggers regulatory mechanisms to decrease cortisol levels — also failed to suppress high levels of the hormone.
Moreover, she showed a significant decrease in renin activity — an enzyme that controls aldosterone production — high levels of aldosterone, and a significantly higher aldosterone/renin ratio, indicative of aldosteronism.
A further CT scan revealed the presence of benign tumors, called adenomas, in both her adrenal glands.
Based on these symptoms, she was diagnosed with non-ACTH-dependent Cushing’s syndrome complicated by primary aldosteronism.
Two surgeries needed to remove woman’s adrenal gland tumors, nodules
The adenoma in her left adrenal gland was removed by robot-assisted laparoscopic surgery, a minimally invasive procedure giving a surgeon access to abdominal organs without large incisions. A nodule close to the adenoma was also removed.
Laboratory analysis found the adenoma expressed CYP11B1, an enzyme that helps produce cortisol, while the nodule expressed CYP11B2, an enzyme that helps produce aldosterone.
However, the patient’s hypertension, fatigue, and weight gain continued after the surgery. She also developed purple stretch marks on the lower abdomen, groin area, and inner thighs, alongside systemic joint pain.
A CT scan now revealed an adenoma in the right adrenal gland. It was removed and found to express both CYP11B1 and CYP11B2, meaning it secreted both cortisol and aldosterone.
After the second surgery, symptoms consistent with Cushing’s syndrome stopped. At a follow-up appointment six months later, she had normal blood pressure, and blood levels of cortisol and aldosterone within normal ranges.
In both adrenal glands, aldosterone-producing micronodules close to the adenomas were detected. According to the researchers, their existence raises a possible risk of primary aldosteronism relapse, necessitating continued checkups.
“This case highlights the need for [aldosterone- and cortisol-producing adenoma] screening,” the researchers concluded, adding that “the complicated pathological features of these cases impose challenges to our understanding of this disease.”